Background: Clinical manifestations of hereditary spherocytosis can be controlled by splenectomy. The use of this procedure has been restricted due to concerns regarding exposure of patients to a lifelong risk of overwhelming infections. Subtotal splenectomy, which removes 85-90% of the enlarged spleen, is a logical alternative. In the first cases performed by laparoscopy we have chosen to preserve the upper pole. However, this technique showed some disadvantages, especially concerning the correct intraoperative evaluation of the splenic remnant volume. Therefore, we developed a new variant of the procedure by preserving the lower pole of the spleen.
Methods: Based on the authors' experience in laparoscopy (176 laparoscopic splenectomies), 10 laparoscopic subtotal splenectomies were performed in patients with hereditary microspherocytosis, preserving either the upper or the lower splenic pole.
Results: Patient age ranged between 5 and 35 years. The mean volume of the remnant spleen was 41.4 cm3. There were no complications, and no transfusions were needed. Follow-up for 1-30 months was available.
Conclusions: Subtotal splenectomy appears to control hemolysis while maintaining splenic function. The laparoscopic approach is safe and effective and should be considered the procedure of choice in hereditary microspherocytosis. Laparoscopic subtotal splenectomy presents an advantage over open subtotal splenectomy, resulting in decreased blood loss, shorter hospital stay, no conversions, fewer operative and postoperative complications, and excellent remission rates. On the basis of our experience, the preservation of the lower pole of the spleen seems to be a first-line option for the optimal evaluation of the residual splenic mass.